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Abstract
Copper-64 studies are presented of 2 patients with non-Wilsonian movement disorder
and with abnormal copper handling. Both patients differred from the usual phenotype
of non-Wilsonian low copper movement disorder as they had choreiform movement disorders
with an onset in the first decade; one patient lacked significant intellectual impairment.
Both patients had reduced serum total copper and marginal free copper and caeruloplasmin
levels, and both patients were capable of incorporating 64Cu2+ into caeruloplasmin but the second case did so at markedly reduced level. Both showed
slightly increased basal and stimulated urinary copper loss compared to normal controls
with the rate in patient 1 being capable of leading to copper depletion. Liver copper
content was normal in both cases. These 2 patients add to the reports of cases with
copper deficiency and movement disorder in whom copper chelation therapy is unlikely
to be beneficial.
Keywords
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Article info
Publication history
Accepted:
March 22,
1993
Received in revised form:
March 16,
1993
Received:
September 16,
1992
Identification
Copyright
© 1993 Published by Elsevier Inc.